A rare case of primary malignant small cell carcinoma combined with urothelial cell carcinoma in the ureter

Abstract

BackgroundExtrapulmonary small cell carcinomas have been reported in a variety of organs, and their incidence in the genitourinary tract is second only to that in the gastrointestinal tract. To date, however, only a few cases of small cell carcinoma of the ureter have been reported. Because the extreme rarity of this type of carcinoma, its clinical behaviour, diagnostic methods, and effective treatment modalities have not yet been determined.Case presentationA 59-year-old man presented with a 1-month history of painless gross haematuria. Urine cytopathology revealed a urothelial carcinoma and computed tomography revealed left hydronephroureterosis with a distal ureteral stone and a mildly enhanced fungating mass just below the stone-impacted site. The preoperative TNM stage was T2N0M0. The patient underwent simultaneous diagnostic ureterorenoscopy and left laparoscopic nephroureterectomy with bladder cuff resection. Gross examination showed a 3.5 × 3.0 × 0.8 cm white, partly yellow mass in the left distal ureter. Light microscopy showed a small cell carcinoma, overlaid on a urothelial carcinoma in situ, invading the ureter and external lateral resection margins. The small cell carcinoma was diffusely positive for neuron-specific enolase, and exhibited focal positivity for CD 56, synaptophysin, chromogranin and cytokeratin 20. The patient was treated with adjuvant chemotherapy, consisting of cisplatin and etoposide, and radiation therapy, and has been well, without evidence of tumour recurrence or metastasis in the 10 months after surgery.ConclusionSmall cell carcinoma of the ureter is rare. Although its clinical behaviour and diagnostic modalities have not been determined and it has yet to be diagnosed immunohistopathologically, multimodality treatment including surgery, chemotherapy and radiotherapy may improve patient survival.

Clinicopathologic characteristics, therapy and outcomes of patients with primary ureteral small cell carcinoma: a case series and systematic review of the literature

“…Concerning the risk factors, previous studies suggested that tobacco exposure might be the principal risk factor for both EPSCC and UTUC. 31 However, only 8 patients from previous studies were reported smokers and none of our 4 patients had a history of smoking. Although the extent to which tobacco use represents a risk factor remains uncertain, our findings indicate that smoking may not be particularly important for the etiology of ureteral SCC.…”

Primary Neuroendocrine Tumors of the Ureter: A Short Review

“…In contrast, urine sediment frequently reveals different degrees of hematuria and leukocyturia. 6,7,[17][18][19] If obstruction is complete, urine cultures may show growth of bacteria that are not the usual uropathogens. 7 Cytology specimens can demonstrate malignant neuroendocrine cells, especially if obtained during an endoscopic study.…”

‘Primary small cell carcinoma of tonsil: An extreme rarity.’

“…Small cell NECs are most commonly seen in lungs and have been reported to occur in some extra-pulmonary sites, mainly in alimentary and genitourinary system [6] A limited number of reports have described small cell NECs arising from the head and neck region and shows the relative propensity for larynx, followed by salivary glands and sinonasal region [2]. Small cell NEC that primarily occurs in tonsil is extremely rare.…”